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cardiomyopathy pediatric

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https://www.readbyqxmd.com/read/28724804/pediatric-dilated-cardiomyopathy-hearts-display-a-unique-gene-expression-profile
#1
Philip D Tatman, Kathleen C Woulfe, Anis Karimpour-Fard, Danielle A Jeffrey, James Jaggers, Joseph C Cleveland, Karin Nunley, Matthew Rg Taylor, Shelley D Miyamoto, Brian L Stauffer, Carmen C Sucharov
Our previous work showed myocellular differences in pediatric and adult dilated cardiomyopathy (DCM). However, a thorough characterization of the molecular pathways involved in pediatric DCM does not exist, limiting the development of age-specific therapies. To characterize this patient population, we investigated the transcriptome profile of pediatric patients. RNA-Seq from 7 DCM and 7 nonfailing (NF) explanted age-matched pediatric left ventricles (LV) was performed. Changes in gene expression were confirmed by real-time PCR (RT-PCR) in 36 DCM and 21 NF pediatric hearts and in 20 DCM and 10 NF adult hearts...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28724792/pediatric-and-adult-dilated-cardiomyopathy-represent-distinct-pathological-entities
#2
Meghna D Patel, Jayaram Mohan, Caralin Schneider, Geetika Bajpai, Enkhsaikhan Purevjav, Charles E Canter, Jeffrey Towbin, Andrea Bredemeyer, Kory J Lavine
Pediatric dilated cardiomyopathy (DCM) is the most common indication for heart transplantation in children. Despite similar genetic etiologies, medications routinely used in adult heart failure patients do not improve outcomes in the pediatric population. The mechanistic basis for these observations is unknown. We hypothesized that pediatric and adult DCM comprise distinct pathological entities, in that children do not undergo adverse remodeling, the target of adult heart failure therapies. To test this hypothesis, we examined LV specimens obtained from pediatric and adult donor controls and DCM patients...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28719387/pediatric-mitochondrial-diseases-and-the-heart
#3
Gregory M Enns
PURPOSE OF REVIEW: Mitochondrial disorders are an increasingly recognized cause of heart dysfunction, with the primary manifestations being cardiomyopathy and conduction defects. This review focuses on the complex genetics of mitochondrial disease and recently discovered conditions that affect mitochondrial function. RECENT FINDINGS: Next-generation sequencing techniques, especially whole-exome sequencing, have led to the discovery of a number of conditions that cause mitochondrial dysfunction and subsequent cardiac abnormalities...
July 17, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28699125/uptake-of-predictive-genetic-testing-and-cardiac-evaluation-for-children-at-risk-for-an-inherited-arrhythmia-or-cardiomyopathy
#4
Susan Christian, Joseph Atallah, Robin Clegg, Michael Giuffre, Cathleen Huculak, Tara Dzwiniel, Jillian Parboosingh, Sherryl Taylor, Martin Somerville
Predictive genetic testing in minors should be considered when clinical intervention is available. Children who carry a pathogenic variant for an inherited arrhythmia or cardiomyopathy require regular cardiac screening and may be prescribed medication and/or be told to modify their physical activity. Medical genetics and pediatric cardiology charts were reviewed to identify factors associated with uptake of genetic testing and cardiac evaluation for children at risk for long QT syndrome, hypertrophic cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy...
July 11, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28694399/familial-hypertrophic-cardiomyopathy-a-case-with-a-new-mutation-in-the-mybpc3-gene
#5
Olgu Hallıoğlu Kılınç, Dilek Giray, Atıl Bişgin, Sevcan Tuğ Bozdoğan, Derya Karpuz
Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease with variable clinical features that is inherited as autosomal dominant with variable penetrance. Recent developments in genetics of hereditary cardiomyopathy have not only enlightened many points about pathogenesis, but have also provided great benefit to diagnostic approaches of clinicians. Heterozygous mutation of c3691-3692insTTCA in MYBPC3 gene was identified in a pediatric patient with diagnosis of hypertrophic cardiomyopathy at clinic...
July 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28669108/novel-genetic-variants-in-bag3-and-tnnt2-in-a-swedish-family-with-a-history-of-dilated-cardiomyopathy-and-sudden-cardiac-death
#6
Eva Fernlund, A Wålinder Österberg, E Kuchinskaya, M Gustafsson, K Jansson, C Gunnarsson
Familial dilated cardiomyopathy is a rare cause of dilated cardiomyopathy (DCM), especially in childhood. Our aim was to describe the clinical course and the genetic variants in a family where the proband was a four-month-old infant presenting with respiratory problems due to DCM. In the family, there was a strong family history of DCM and sudden cardiac death in four generations. DNA was analyzed initially from the deceased girl using next-generation sequencing including 50 genes involved in cardiomyopathy...
July 1, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28660071/comparison-of-two-dimensional-strain-analysis-using-vendor-independent-and-vendor-specific-software-in-adult-and-pediatric-patients
#7
Shafkat Anwar, Kazuaki Negishi, Allen Borowszki, Patrick Gladding, Zoran B Popović, Francine Erenberg, James D Thomas
INTRODUCTION: Two-dimensional strain analysis is a powerful analysis modality, however, clinical utilization has been limited by variability between different analysis systems and operators. We compared strain in adults and children using vendor-specific and vendor-independent software to evaluate variability. METHODS: One hundred and ten subjects (50/110 pediatric, 80/110 normal left ventricular function) had echocardiograms with a General Electric ultrasound scanner between September 2010 and January 2012...
January 2017: JRSM Cardiovascular Disease
https://www.readbyqxmd.com/read/28653469/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-chest-pain
#8
Jimmy C Lu, Manish Bansal, Sarina K Behera, Jeffrey R Boris, Brian Cardis, John S Hokanson, Bahram Kakavand, Roy Jedeikin
OBJECTIVE: As part of the American College of Cardiology Adult Congenital and Pediatric Cardiology Section effort to develop quality metrics (QMs) for ambulatory pediatric practice, the chest pain subcommittee aimed to develop QMs for evaluation of chest pain. DESIGN: A group of 8 pediatric cardiologists formulated candidate QMs in the areas of history, physical examination, and testing. Consensus candidate QMs were submitted to an expert panel for scoring by the RAND-UCLA modified Delphi process...
June 27, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28619384/risk-stratification-to-determine-the-impact-of-induction-therapy-on-survival-rejection-and-adverse-events-after-pediatric-heart-transplant-a-multi-institutional-study
#9
Chesney Castleberry, Elizabeth Pruitt, Rebecca Ameduri, Kenneth Schowengerdt, Erik Edens, Nancy Hagin, James K Kirklin, David Naftel, Simon Urschel
BACKGROUND: Induction therapy is increasingly being used in pediatric heart transplantation. General versus risk-adapted use remains controversial. We aimed to determine the impact of induction therapy on outcomes after stratifying patients by diagnosis and risk. METHODS: The Pediatric Heart Transplant Study (PHTS) database was used to identify patients (age ≤18 years) who underwent transplantation between January 1, 2001 and December 31, 2014. Patients were excluded if they survived <48 hours or received multiple induction agents...
May 11, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28568969/clinical-significance-of-anti-hla-antibodies-associated-with-ventricular-assist-device-use-in-pediatric-patients-a-united-network-for-organ-sharing-database-analysis
#10
H Sonali Magdo, Kurt R Schumacher, Sunkyung Yu, Robert J Gajarski, Joshua M Friedland-Little
While VAD use in pediatric patients has previously been associated with anti-HLA antibody production, the clinical significance of these antibodies is unclear. We investigated the clinical impact of anti-HLA antibodies associated with VAD use in a large cohort of pediatric HTx recipients. From 2004 to 2011, pediatric cardiomyopathy patients post-HTx (N=1288) with pre-HTx PRA levels were identified from the United Network for Organ Sharing database. PRA levels were compared between VAD patients and those with no history of MCS...
August 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28565750/myocardial-fibrosis-in-congenital-and-pediatric-heart-disease
#11
Jing Tian, Xinjiang An, Ling Niu
Cardiac fibrosis is a common phenomenon in different types of heart diseases, such as ischemic heart disease, inherited cardiomyopathy mutations, diabetes, and ageing and is associated with morbidity and mortality. Increased accumulation of extracellular matrix (ECM) that impacts cardiac function, is the underlying cause of fibrotic heart disease. There are four different types of cardiac fibrosis, including, reactive interstitial fibrosis, replacement fibrosis, infiltrative interstitial fibrosis and endomyocardial fibrosis...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28550929/arrhythmia-and-clinical-cardiac-findings-in-children-with-anderson-fabry-disease
#12
Hunter C Wilson, Robert J Hopkin, Peace C Madueme, Richard J Czosek, Laurie A Bailey, Michael D Taylor, John L Jefferies
Anderson-Fabry Disease (AFD) is a lysosomal storage disorder that results in progressive cardiovascular hypertrophy, scarring, and arrhythmia burden; yet, the early cardiac phenotype of AFD is still poorly defined. To further characterize early cardiac features in AFD, we evaluated electrocardiographic and clinical findings contained in a local cohort of pediatric AFD patients and arrhythmia data in children enrolled in the Fabry Registry. Twenty-six local patients aged <18 years were identified (average age 9...
July 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28472305/recessive-taf1a-mutations-reveal-ribosomopathy-in-siblings-with-end-stage-pediatric-dilated-cardiomyopathy
#13
Pamela A Long, Jeanne L Theis, Yu-Huan Shih, Joseph J Maleszewski, Patrice C Abell Aleff, Jared M Evans, Xiaolei Xu, Timothy M Olson
Non-ischemic dilated cardiomyopathy (DCM) has been recognized as a heritable disorder for over 25 years, yet clinical genetic testing is non-diagnostic in > 50% of patients, underscoring the ongoing need for DCM gene discovery. Here, whole exome sequencing uncovered a novel molecular basis for idiopathic end-stage heart failure in two sisters who underwent cardiac transplantation at three years of age. Compound heterozygous recessive mutations in TAF1A, encoding an RNA polymerase I complex protein, were associated with marked fibrosis of explanted hearts and gene-specific nucleolar segregation defects in cardiomyocytes, indicative of impaired ribosomal RNA synthesis...
May 2, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28453909/cardiotoxicity-and-cardiomyopathy-in-children-and-young-adult-survivors-of-hematopoietic-stem-cell-transplant
#14
REVIEW
Seth J Rotz, Thomas D Ryan, Joel Hlavaty, Stephen A George, Javier El-Bietar, Christopher E Dandoy
Cardiomyopathy is common in long-term survivors of pediatric hematopoietic stem cell transplant (HSCT). Events occurring before and after HSCT when combined with specific insults during HSCT likely contribute to long-term risk. Strategies for detecting subclinical cardiomyopathy prior to patients developing overt heart failure are under investigation. Changes in HSCT preparative regimens and cardioprotective medications administered during chemotherapy may alter the risk for cardiomyopathy. Interventions in long-term survivors such as lifestyle modification and cardioactive medications are of increasing importance...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28452856/administration-of-dexrazoxane-improves-cardiac-indices-in-children-and-young-adults-with-acute-myeloid-leukemia-aml-while-maintaining-survival-outcomes
#15
Nathan J Schloemer, Molly Brickler, Raymond Hoffmann, Amy Pan, Pippa Simpson, Vanessa McFadden, Joseph Block, Richard L Tower, Michael J Burke
Anthracycline-induced cardiotoxicity remains a significant contributor to late morbidity/mortality in children and young adults with acute myeloid leukemia (AML). The cardioprotectant dexrazoxane can be used as prophylaxis to diminish risk for cardiomyopathy but whether it affects risk of relapse in pediatric AML is unclear. Our institution adopted the use of dexrazoxane before anthracyclines administration for all oncology patients in 2011. We compared patients with AML (ages, 0 to 21 y) who received or did not receive dexrazoxane during the years 2008 to 2013...
April 27, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28429690/-our-experience-in-the-diagnosis-and-treatment-of-postural-orthostatic-tachycardia-syndrome-vasovagal-syncope-and-inappropriate-sinus-tachycardia-in-children
#16
Sezen Ugan Atik, Reyhan Dedeoğlu, Aida Koka, Funda Öztunç
OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28424442/clinical-improvement-and-no-further-need-of-transplant-after-closure-of-pda-with-transcatheter-approach-in-an-end-stage-heart-failure-patient-with-hypertrabeculation
#17
Serhat Koca, Feyza Ayşenur Paç, Ajda Mutlu Mıhçıoğlu, Vedat Kavurt, Denizhan Bağrul
Although heart failure is managed medically most of the time, heart transplantation is still last resort for selected end-stage heart failure patients with noncompaction cardiomyopathy. Presently described for the first time is case of pediatric patient with noncompaction cardiomyopathy who was initially referred to our hospital for heart transplant but underwent PDA repair and improved clinically without need for heart transplant.
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28424438/using-cardiovascular-imaging-modalities-to-determine-cardiac-disorders-before-starting-sports-activities
#18
Özgür Ceylan, Timur Meşe, Alper Hazım Gürsu
OBJECTIVE: We re-examined children who had previously been declared eligible to participate in competitive sports activities for cardiac disorders, using cardiac investigation protocol. METHODS: Total of 250 children (224 males [89.6%], and 26 females [10.4%]) between the ages of 8 and 17 years who had just started or were already engaged in sports activities were included in the study. Participants had detailed physical examination evaluated by a pediatric cardiologist...
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28401251/delayed-myocardial-enhancement-in-pediatric-hypertrophic-cardiomyopathy-correlation-with-lv-function-echocardiography-and-demographic-parameters
#19
Sonia El Saiedi, Noha Hossam Behairy, Ahmed Kharabish, Reem Esmail, Zeinab Salah Seliem, Mervat Shafik, Wesam El Mozy
Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI)...
April 11, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28382424/plasma-exchange-for-the-patients-with-dilated-cardiomyopathy-in-children-is-safe-and-effective-in-improving-both-cardiac-function-and-daily-activities
#20
Takeshi Moriguchi, Keiichi Koizumi, Kenichi Matsuda, Norikazu Harii, Junko Goto, Daiki Harada, Hisanori Sugawara, Minako Hoshiai, Hiroaki Kise, Akiyasu Baba
Autoantibodies against cardiac proteins play an important role in the development of dilated cardiomyopathy (DCM). The efficacy and safety of apheresis such as immunoadsorption (IA) or plasma exchange (PE) to remove such antibodies have been reported in adult DCM patients. However, apheresis for pediatric DCM has not been performed because of technical difficulty due to relatively low blood volume and instability of hemodynamics. As we have experiences of preforming apheresis on hemodynamically unstable children, we have preformed ten courses of PE on seven child DCM patients including both patients in chronic and acute phase to assess the safety and efficacy to PE...
April 5, 2017: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
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